ENDOSCOPIC CONTROL OF POLYP BURDEN WITH SMALL-BOWEL ENDOSCOPY IN PEUTZ-JEGHERS SYNDROME. A THIRTY-YEAR EXPERIENCE IN A TERTIARY REFERRAL CENTER

Aims We investigated the clinical outcome of small-bowel endoscopy as part of an oncological surveillance program in a large series of PJS patients.

Methods PJS patients were consecutively enrolled from our dedicated outpatient-clinic for inherited polyposis syndromes. We selected patients who underwent enteroscopy (push, single, double-balloon or intraoperative enteroscopy) from 1990 to 2019. The indication for enteroscopy was detection of >1.5cm polyps at a previous imaging study such as VCE, MRE or small-bowel series/enteroclysis. The outcome of enteroscopy, the rate of adverse events, follow-up data and oncological data were recorded.

Results 26 patients (16 M, 10 F) were included (mean age 36, range 11-54). 65 enteroscopies were performed: 9 intraoperative (IOE), 16 push, 40 device-assisted enteroscopy (DAE). Excluding IOE, 43 procedures were performed with anterograde approach. In total, 274 small-bowel polyps >5mm were removed, of which 2 were HGD adenomas. In IOE, 1 jejunal bleeding and 1 small-bowel perforation were recorded (2/9 procedures, 22%), both related to the surgical procedure; conversely in DAE, only 2 minor polypectomy-related bleedings and 1 pneumothorax were recorded (3/56, 5,3%). On the basis of the patients’ remote clinical history, 18/26 patients (69,2%) underwent emergent surgery (mainly for intestinal occlusion) before being subjected to enteroscopy; conversely during the follow-up after enteroscopy (mean 46 months, range 5-194) only 2/21 (9,5%) underwent emergent surgery. 5 patients were lost at follow-up. 11/26 patients (42,3 %) developed extra-GI neoplasias during the follow-up but no GI cancers (2 GI cancers were detected before starting enteroscopies and were treated surgically). 4 patients died during follow-up because of extra-GI cancers.

Conclusions A systematic endoscopic approach to small-bowel polyps, to achieve and maintain low polyp burdens, appears to be useful in reducing the rate of urgent surgical interventions. DAE has a good safety profile compared to IOE. At present, extra-GI neoplasias remain the main issue affecting these patients.